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Cancer Cytopathology Aug 2013Acinar cell neoplasms of the pancreas are rare but when encountered, the diagnosis is often established based on cytology specimens. Diagnostic accuracy is important...
BACKGROUND
Acinar cell neoplasms of the pancreas are rare but when encountered, the diagnosis is often established based on cytology specimens. Diagnostic accuracy is important because acinar cell carcinomas are aggressive yet may mimic tumors with different outcomes and management.
METHODS
The authors identified all patients with a diagnosis of acinar cell neoplasm in the institutional database; assessed cytomorphology and immunocytochemistry for trypsin, chymotrypsin, synaptophysin, chromogranin A, and MIB-1; and compared all cytology and final histological diagnoses for diagnostic discrepancies.
RESULTS
Cytological features were described for 16 histologically proven malignant acinar cell neoplasms: acinar cell carcinoma (8 cases), mixed acinar-neuroendocrine carcinoma (6 cases), mixed acinar-ductal carcinoma (1 case), and pancreatoblastoma (1 case).The majority of aspirates from acinar cell cystadenomas were nondiagnostic or negative (5 of 6 cases; 83%). Acinar and neuroendocrine differentiation that was detected by immunocytochemistry in >20% of tumor cells was found to be correlated with mixed acinar-neuroendocrine carcinoma histology. Cytohistological correlation included 32 patients with 17 discordant diagnoses (53%). The following preoperative cytology diagnoses proved to be acinar cell neoplasms on resection: neuroendocrine tumor (5 cases), adenocarcinoma (5 cases), atypical ductal cells (2 cases), solid pseudopapillary neoplasm, and hepatocellular carcinoma. Three aspirates diagnosed as acinar cell carcinoma by cytology proved to be chronic pancreatitis (2 cases) and ductal adenocarcinoma (1 case).
CONCLUSIONS
Acinar cell carcinoma has a distinctive cytological appearance but is frequently misdiagnosed on cytology. Immunocytochemistry is useful for identifying acinar differentiation.
Topics: Adult; Carcinoma, Acinar Cell; Child; Female; Humans; Immunohistochemistry; Immunophenotyping; Male; Middle Aged; Pancreatic Neoplasms
PubMed: 23408736
DOI: 10.1002/cncy.21279 -
Iranian Red Crescent Medical Journal Feb 2013Hematopoietic stem cell transplantation (HSCT) is a new window to therapy of many diseases. From March 1991 through April 2011, a total of 3237 HSCT were performed in...
BACKGROUND
Hematopoietic stem cell transplantation (HSCT) is a new window to therapy of many diseases. From March 1991 through April 2011, a total of 3237 HSCT were performed in the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. Here we report 20 years experience of HSCT.
OBJECTIVES
Our strategy and aim include the protraction of cytogenetic and molecular biological diagnostic tests, the expansion of the first Iranian Cord Blood Bank (ICBB) and development of the first Iranian Stem Cell Donor Program (ISCDP), and improvement the researches in new therapeutic fields.
PATIENTS AND METHODS
Totally, 3237 patients were undergone HSCT. Of these transplants, 2205 were allogeneic stem cell transplantation, 1016 autologous and 16 syngeneic. Among 2205 patients who were undergone allogenic-HSCT, 34 received cord blood stem cells as stem cell source for transplantation. It is important to point out that cord blood bank at our center provides reliable storage of cord blood stem cells for our patients. Stem cell transplantation was performed for treatment of various diseases such as acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, beta-thalassemia major, sickle- cell thalassemia, sickle- cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combined immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. Also, we had 220 cellular therapies for post-myocardial infarction, multiple sclerosis, cirrhosis, head of femur necrosis, Diabetes Mellitus and GvHD treatment. 45 patients were undergone retransplantation in this center.
RESULTS
About 78.2% of the patients (2530 of 3237) remained alive between one to 211 months after stem cell transplantation. Nearly, 21.8% (707) of our patients died after stem cell transplantation. The main causes of death were relapse, infection, hemorrhagic cystitis, graft-versus- host disease and etc.
CONCLUSIONS
In Iran, HSCT has been successfully adapted in routine clinical care. Recently, new methods such as double cord blood and haploidentical transplantation have been used to treat many life-threatening diseases.
PubMed: 23682320
DOI: 10.5812/ircmj.1915 -
Pediatric Blood & Cancer Jun 2023Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic...
Primary pancreatic tumors in children are rare with an overall age-adjusted incidence of 0.018 new cases per 100,000 pediatric patients. The most prevalent histologic type is the solid pseudopapillary neoplasm, followed by pancreatoblastoma. This paper describes relevant imaging modalities and presents consensus-based recommendations for imaging at diagnosis and follow-up.
Topics: Child; Humans; Surface Plasmon Resonance; Pancreatic Neoplasms; Tomography, X-Ray Computed; Carcinoma, Papillary; Pancreas
PubMed: 36215203
DOI: 10.1002/pbc.29975 -
Frontiers in Oncology 2024Pancreatoblastoma (PB) is a rare malignant pancreatic epithelial tumor that mostly occurs in children and occasionally occurs in adults. The tumor has acinar cell...
Pancreatoblastoma (PB) is a rare malignant pancreatic epithelial tumor that mostly occurs in children and occasionally occurs in adults. The tumor has acinar cell differentiation and squamous corpuscles/squamous epithelial islands, which are frequently separated by fibrous bundles. Familial adenomatous polyposis (FAP) is an autosomal dominant inherited disease characterized by the presence of numerous adenomatous polyps in the colon and rectum. Cases of pancreatoblastoma combined with familial adenomatous polyposis (FAP) are rarely reported. A review of a rare case of adult pancreatoblastoma with atypical histological morphology combined with familial adenomatous polyposis is presented herein. In this case, the patient was first diagnosed with familial adenomatous polyposis and subsequently found to have pancreatoblastoma 1 year and 3 months later. This suggests pancreatoblastoma may occur in patients with familial adenomatous polyposis or a family history of the condition, indicating a possible association between the two tumors. Therefore, pancreatoblastoma should be included in a differential diagnosis for FAP patients with a pancreatic mass. The final diagnosis of pancreatoblastoma depends on the pathological diagnosis. Acinar-like cells and squamous corpuscles/squamous epithelial cell islands under light microscopy are the key diagnostic points. This case report also can improve the awareness of clinicians, radiologists, and pathologists on the presence of rare tumor-adult pancreatoblastoma in patients with familial adenomatous polyposis.
PubMed: 38482206
DOI: 10.3389/fonc.2024.1346964 -
Oncotarget Feb 2018During next generation sequencing (NGS) analysis, many missense mutations were found in a well-known oncogene, many of which were variant of uncertain significance...
During next generation sequencing (NGS) analysis, many missense mutations were found in a well-known oncogene, many of which were variant of uncertain significance mutations. We recently treated an adult patient with pancreatoblastoma by chemotherapy. Using an NGS cancer panel, we found a previously unreported missense mutation in the 1835 codon of the () gene. We also found a heterogeneous mutation in the 1835 codon of the gene in the patient's germline by Sanger sequencing. Although this patient did not have a history of familial adenomatous polyposis, functional analysis suggested the R1835G mutant showed attenuated repression of Wnt/β-catenin signaling activity. This is the first report showing a novel missense mutation involved in the onset of adult pancreatoblastoma.
PubMed: 29535845
DOI: 10.18632/oncotarget.24017 -
Applied Immunohistochemistry &... Oct 2011Pancreatic and duodenal homeobox (Pdx1) is a homeobox transcription factor required for the embryonic development of the pancreas. Pdx1 expression has been earlier...
Pancreatic and duodenal homeobox (Pdx1) is a homeobox transcription factor required for the embryonic development of the pancreas. Pdx1 expression has been earlier identified in pancreatic ductal adenocarcinomas and endocrine neoplasms. This study characterizes Pdx1 protein expression in pancreatic precursor lesions and neoplasms, including pancreatic intraepithelial neoplasia (PanIN, n=32), intraductal papillary mucinous neoplasm (IPMN, n=88), mucinous cystic neoplasm (MCN, n=3), acinar cell carcinoma (ACC, n=8), pancreatic endocrine neoplasm (PEN, n=44), pancreatoblastoma (PB, n=1), solid pseudopapillary neoplasm (n=8), invasive ductal adenocarcinoma (n=67), and nondysplastic ductal epithelium. A mouse monoclonal antibody for Pdx1 was used to examine archived surgical pathology cases and tissue microarrays containing >655 tissue cores from more than 250 pancreatic specimens. Immunohistochemical labeling for Pdx1 was performed using standard methods and scored for percentage and intensity of nuclear labeling. Among non-neoplastic pancreatic tissues, Pdx1 nuclear protein was expressed in islet cells, cells of the centroacinar cell compartment, and non-neoplastic ductal epithelium. No expression of Pdx1 was seen in non-neoplastic acinar cells. Among pancreatic neoplasms, Pdx1 consistently labeled >50% of the tumor cells in 87.5% of ACC cases and 38.6% of PEN cases. Pdx1 expression was variable in invasive ductal adenocarcinoma and precursor lesions of ductal adenocarcinomas (PanIN, IPMN, and MCN). A single case of PB was examined and it showed Pdx1 in the acinar component, but no expression in squamoid nests. Solid pseudopapillary neoplasms did not express Pdx1. This study shows Pdx1 expression in precursor lesions of ductal adenocarcinomas, PEN, ACC, and a case of PB. In the immunohistochemical evaluation of neoplasms of the pancreas, Pdx1 expression is not a finding specific to PENs and ductal adenocarcinomas, but also occurs in precursor lesions (PanIN, IPMN, MCN) and other neoplasms of the pancreas.
Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Carcinoma, Pancreatic Ductal; Cells, Cultured; Early Detection of Cancer; Female; Gene Expression Regulation, Neoplastic; Homeodomain Proteins; Humans; Immunohistochemistry; Male; Middle Aged; Pancreatic Neoplasms; Trans-Activators
PubMed: 21297446
DOI: 10.1097/PAI.0b013e318206d958 -
Oncology Letters Oct 2017Pancreatic cancer is classified as ductal, acinar, neuroendocrine carcinoma or pancreatoblastoma. Ductal and acinar cells derive from exocrine glands and neuroendocrine...
Pancreatic cancer is classified as ductal, acinar, neuroendocrine carcinoma or pancreatoblastoma. Ductal and acinar cells derive from exocrine glands and neuroendocrine cells from endocrine glands; however, mixed acinar-neuroendocrine-ductal carcinoma has different histological carcinomas coexisting within a nodule. The mixed pancreatic carcinoma forms from different developmental origins and therefore requires investigation. The current case report presents a 50-year-old male who had a tumor within the body of the pancreas. Pathological examination clarified the tumor as a mixed acinar-neuroendocrine-ductal carcinoma. The ductal and acinar/neuroendocrine tumor components were isolated using laser-capture microdissection, and next-generation sequencing analysis was performed. Consequently, frameshift (p.N210fs) and missense (p.G12R) mutations were identified in both ductal and acinar/neuroendocrine tumors. These results suggested a pancreatic mixed acinar-neuroendocrine-ductal carcinoma was derived from a founder tumor clone, and supports the notion that a founder tumor clone may differentiate and transform into a diverse histological type and form a pancreatic mixed carcinoma.
PubMed: 29085438
DOI: 10.3892/ol.2017.6786 -
Cureus Jan 2020Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is...
Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is challenging due to its vague symptoms. The initial diagnosis is made by imaging, afterwards the management is usually by resection of the tumor with or without chemotherapy which depends on the size and grade of the tumor. We report a case of a nine-year-old girl who was diagnosed with pancreotoblastoma and underwent complete resection with chemotherapy.
PubMed: 32015939
DOI: 10.7759/cureus.6779 -
Journal of Indian Association of... 2018Whipple's pancreaticoduodenectomy (WPD) is rarely required in children. However, WPD is the only option with pathologies involving the head of the pancreas requiring...
PURPOSE
Whipple's pancreaticoduodenectomy (WPD) is rarely required in children. However, WPD is the only option with pathologies involving the head of the pancreas requiring surgical excision. The objective of our study was to review our experience with WPD performed on children.
MATERIALS AND METHODS
A retrospective analysis of case records was conducted on all patients <18 years of age, who underwent WPD at our center over the last 20 years. Data regarding demographics, signs, and symptoms at presentation, diagnostic imaging and procedures, pathologic reports, surgical and medical treatment, and follow-up were collected to study the indications and safety and outcomes of WPD in children.
RESULTS
Five patients had been planned for a WPD during the study (1995-2015); but in one patient, the procedure was abandoned, the rest four patients formed the study group. Male to female ratio was 3:1. Median age at the time of surgery was 9 years (11 months-12 years). The most common presentation was obstructive jaundice (50%, 2/4). Radiological imaging was able to accurately predict the surgical procedure required in all except one case. The mean operating time was 205 min (180-240 min). There were no intraoperative complications. The mean intraoperative blood loss was 85 mL (20-150 mL). The youngest patient requiring WPD was an 11-month-old child. Oral feeding was established by the 7 postoperative day (range 5-7 days) in all cases. There were no cases of anastomotic leak or pancreatic or jejunal fistulae. One patient developed features of subacute intestinal obstruction after discharge and required re-exploration. There was no intra- or post-operative mortality.
CONCLUSION
WPD is safe and efficacious procedure in a selected group of children. The overall efficacy of surgical treatment combined with the relatively low severity of complications leads us to recommend WPD in children when indicated.
PubMed: 30443117
DOI: 10.4103/jiaps.JIAPS_35_18 -
CytoJournal 2014The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS) guided...
The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS) guided fine-needle aspiration (FNA) biopsy, techniques of EUS-FNA, terminology and nomenclature of pancreatobiliary disease, ancillary testing and post-biopsy treatment and management. All documents are based on the expertise of the authors, a review of the literature, discussion of the draft document at several national and international meetings over an 18 month period and synthesis of online comments of the draft document on the Papanicolaou Society of Cytopathology web site [www.papsociety.org]. This document selectively presents the results of these discussions and focuses on a proposed standardized terminology scheme for pancreatobiliary specimens that correlate cytological diagnosis with biological behavior and increasingly conservative patient management of surveillance only. The proposed terminology scheme recommends a six-tiered system: Non-diagnostic, negative, atypical, neoplastic [benign or other], suspicious and positive. Unique to this scheme is the "neoplastic" category separated into "benign" (serous cystadenoma) or "other" (premalignant mucinous cysts, neuroendocrine tumors and solid-pseudopapillary neoplasms (SPNs)). The positive or malignant category is reserved for high-grade, aggressive malignancies including ductal adenocarcinoma, acinar cell carcinoma, poorly differentiated neuroendocrine carcinomas, pancreatoblastoma, lymphoma and metastases. Interpretation categories do not have to be used. Some pathology laboratory information systems require an interpretation category, which places the cytological diagnosis into a general category. This proposed scheme provides terminology that standardizes the category of the various diseases of the pancreas, some of which are difficult to diagnose specifically by cytology. In addition, this terminology scheme attempts to provide maximum flexibility for patient management, which has become increasingly conservative for some neoplasms.
PubMed: 25191517
DOI: 10.4103/1742-6413.133343